3BP2-deficient mice are osteoporotic with impaired osteoblast and osteoclast functions

JUMP TO:

Patients with cherubism, which is a gain-of-function mutation in 3BP2, have cystic lesions with activated osteoclasts and craniofacial abnormalities. 3BP2 is a gene that encodes for SH3-domain binding protein 2 (SH3BP2). In this paper the authors examined 3BP2 deficient mice. Sh3bp2-/-mice developed osteoporosis as a result of reduced bone formation and bone resorption. They also found that that 3BP2 was required for Abl activation in osteoblasts and Src activation in osteoclasts, and demonstrated that the in vitro defect of each cell type was restored by the respective expression of activated forms of these kinases.